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Mantle Cell Lymphoma

Abstract

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Synonyms of Mantle Cell Lymphoma

No synonyms found.

Disorder Subdivisions

No subdivisions found.

General Discussion

Mantle cell lymphoma (MCL) belongs to a group of diseases known as non-Hodgkin's lymphomas, which are related malignancies (cancers) that affect the lymphatic system (lymphomas). Functioning as part of the immune system, the lymphatic system helps to protect the body against infection and disease. It consists of a network of tubular channels (lymph vessels) that drain a thin watery fluid known as lymph from different areas of the body into the bloodstream. Lymph accumulates in the tiny spaces between tissue cells and contains proteins, fats, and certain white blood cells known as lymphocytes.

As lymph moves through the lymphatic system, it is filtered by a network of small structures known as lymph nodes that help to remove microorganisms (e.g., viruses, bacteria, etc.) and other foreign bodies. Groups of lymph nodes are located throughout the body, including in the neck, under the arms (axillae), at the elbows, and in the chest, abdomen, and groin. Lymphocytes are stored within lymph nodes and may also be found in other lymphatic tissues. In addition to the lymph nodes, the lymphatic system includes the spleen, which filters worn-out red blood cells and produces lymphocytes, and the tonsils, which are masses of lymphoid tissue in the throat region that help to fight infection. Lymphatic tissues also include the thymus, a relatively small organ behind the breastbone that is thought to play an important role in the immune system until puberty, as well as the bone marrow, which is the spongy tissue inside the cavities of bones that manufactures blood cells. Lymphatic tissue or circulating lymphocytes may also be located in other regions of the body, such as the skin, small intestine, liver, and other organs. There are two main types of lymphocytes: B-lymphocytes, which may produce specific antibodies to "neutralize" certain invading microorganisms, and T-lymphocytes, which may directly destroy microorganisms or assist in the activities of other lymphocytes.

Mantle cell lymphoma and other cancers of the lymphatic system (lymphomas) result from errors in the production of a lymphocyte or transformation of a lymphocyte into a malignant cell. Abnormal, uncontrolled growth and multiplication (proliferation) of malignant lymphocytes may lead to enlargement of a specific lymph node region or regions; involvement of other lymphatic tissues, such as the spleen and bone marrow; and spread to other bodily tissues and organs, potentially resulting in life-threatening complications. The specific symptoms and physical findings may vary from case to case, depending upon the extent and region(s) of involvement and other factors.

Non-Hodgkin's lymphomas (NHLs) may be broadly classified into lymphomas that arise from abnormal B-lymphocytes (B-cell lymphomas) and those derived from abnormal T-lymphocytes (T-cell lymphomas). Mantle cell lymphoma (MCL) is a B-cell lymphoma that develops from malignant B-lymphocytes within a region of the lymph node known as the mantle zone. NHLs may also be categorized based upon certain characteristics of the cancer cells as seen under a microscope and how quickly they may tend to grow and spread. For example, NHLs may be characterized as "low-grade" (or indolent) lymphomas, which tend to grow slowly and result in few associated symptoms, or "intermediate-" or "high-grade" (aggressive) lymphomas, which typically grow rapidly, requiring prompt treatment. There is some debate concerning whether MCL should be categorized as a slow-growing (indolent) or rapidly-growing (aggressive) lymphoma. Although experts have classified MCL as an aggressive lymphoma, it has been shown to have certain characteristics of indolent lymphoma.

According to various estimates, MCL represents approximately 2 to 7 percent of adult NHLs in the United States and Europe. It primarily affects men over the age of 50 years. Many affected individuals have widespread disease at diagnosis, with involved regions often including multiple lymph nodes, the spleen, and, potentially, the bone marrow, the liver, and/or regions of the digestive (gastrointestinal) tract.

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